The expression of the mitochondrial encoded gene ND4 is downregulated in cystic fibrosis

Abstract: Cystic fibrosis (CF) is a disease produced by mutations in the CFTR channel. We have previously reported that the CFTR chloride transport activity regulates the differential expression of several genes, including SRC. Here we report that MT-ND4, a mitochondrial gene encoding a subunit of t...

Descripción completa

Guardado en:
Detalles Bibliográficos
Autores principales: Valdivieso, Ángel Gabriel, Marcucci, Florencia, Taminelli, Guillermo, González Guerrico, Anatilde, Alvarez, Sergio, Dankert, Marcelo A., Teiber, María Luz, Santa Coloma, Tomás Antonio
Formato: Artículo
Lenguaje:Inglés
Inglés
Publicado: Elsevier 2019
Materias:
FIBROSIS QUISTICA
MITOCONDRIAS
GENES MITOCONDRIALES
GLIBURIDA
CFTR
MT-ND4
CFDE
CFTR(inh)-172
Acceso en línea:https://repositorio.uca.edu.ar/handle/123456789/1456
Aporte de:
Repositorio Institucional de la Universidad Católica Argentina (UCA) de Universidad Católica Argentina
Descripción
Sumario:Abstract: Cystic fibrosis (CF) is a disease produced by mutations in the CFTR channel. We have previously reported that the CFTR chloride transport activity regulates the differential expression of several genes, including SRC. Here we report that MT-ND4, a mitochondrial gene encoding a subunit of the mitochondrial Complex I (mtCx-I), is also a CFTR-dependent gene. A reduced expression of MT-ND4 was observed in CFDE cells (derived from a CF patient) when compared to CFDE cells ectopically expressing wild type CFTR. The differential expression of MT-ND4 in CF was confirmed by PCR. In situ hybridizations of deparaffinized human lung tissue slices derived from wt-CFTR or CF patients also showed downregulation of ND4 in CF. In addition, glibenclamide or CFTR(inh)-172 (CFTR chloride transport inhibitors) reduced MT-ND4 expression in cells expressing wt CFTR. These results suggest that the CFTR chloride transport activity indirectly up-regulates MT-ND4 expression.

Ejemplares similares